Acoustic Neuroma:

An acoustic neuroma is a benign neoplasm which arises from the hearing nerve (8th cranial nerve). An acoustic neuroma can also be called an acoustic schwannoma or neurilemmoma. Acoustic neuromas account for up to 10% of all intracranial tumors and up to 90% of all tumors of the cerebellopontine angle. Females are affected more than males. Symptoms include progressive sensorineural hearing loss, tinnitus, and loss of equilibrium. These tumors may also affect other nerves as they enlarge. Typical treatment is complete surgical excision. As an alternative to surgical removal, stereotactic radiosurgery has successfully controlled these tumors in selected cases.

Adenoid Cystic Carcinoma:

An adenoid cystic carcinoma is a malignant epithelial salivary gland tumor that has a tendency to invade nerves. Adenoid cystic carcinomas represent approximately 12% of all malignant salivary gland tumors. They represent 50% of lacrimal gland neoplasms. There is no sex predilection and they occur over a wide age range. The most common presenting symptom is a mass with or without pain. Cranial nerve dysfunction can been seen as well. Wide local surgical excision achieving negative margins followed by radiation therapy is the treatment plan of choice. This treatment approach to adenoid cystic cancers of the skull base, orbits as well as in the more common head and neck locations has been yielding successful outcomes. Adenoid cystic cancers typically metastasize along nerves and can exhibit long latency periods. For this reason, long term clinical follow up is important as metastases can develop 10 or even 20 years after the primary tumor is resected.

Basaloid Carcinoma:

A basaloid squamous cell carcinoma is an invasive neoplasm in which basaloid cells are associated with either in-situ squamous cell carcinoma, invasive squamous cell carcinoma or with dysplastic squamous epithelium. It is an uncommon variant of squamous cell carcinoma. Symptoms depend on the site of occurrence. Treatment involves radical surgical excision. See squamous cell cancer for further information.

Cholesteatoma:

A cholesteatoma is an invasive condition characterized by the presence of skin that forms a saclike accumulation of keratin within the middle ear space. A cholesteatoma tends to affect more men than women. Cholesteatomas can cause hearing loss, malodorous discharge, and pain. A small percentage of cholesteatomas are congenital. Complete surgical excision is the treatment of choice. If incompletely excised, this condition will continue to progress.

Cholesterol Granuloma:

A cholesterol granuloma represents a foreign body granulomatous response to cholesterol crystals. Cholesterol granulomas are most frequently found in the middle ear in an environment in which hemorrhage has occurred and interference in the drainage and ventilation of the middle ear space is present. Complications may be lethal and may include meningitis, brain abscess or acute mastoiditis. Treatment includes surgical excision or, in cases of petrous apex granulomas, drainage into the sinuses.

Chondroma:

Chondromas are benign tumors of cartilage. Cartilaginous tumors of the head and neck, such as chondromas are uncommon. A chordoma affects males more that females and can occur at any age. The most common head and neck sites of occurrence are the paranasal sinuses, nasal cavity, maxilla, mandible, larynx, palate, pharynx, nasopharynx and ear. Symptoms vary according to the site of involvement, but may include, nasal obstruction, mass, shortness of breath, or hoarseness. Treatment involves conservative but wide surgical removal of the tumor. Recurrences are rare following adequate removal of the tumor.

Chondromyxoid Fibroma:

Chondromyxoid fibromas are benign tumors of cartilaginous origin that can involve the craniofacial and paranasal sinuses. Chondromyxoid fibromas tend to affect men more than women. They occur most commonly in the second or third decade of life. The mandible is the most common site. Symptoms caused by chondromyxoid fibromas may include pain, loose teeth, headache and trismus (difficulty opening the mouth). Surgical resection is the treatment of choice. Local recurrence may occur if incompletely excised.

Chondrosarcoma:

A chondrosarcoma is a malignant tumor of cartilage. Chondrosarcomas are rare in the head and neck region. Symptoms are dependent upon location but can include airway obstruction and bleeding. In the sinonasal region, symptoms can include changes in dentition, visual change, pain, and neural deficits. Treatment includes surgical excision with the possible addition of chemotherapy.

Chordoma:

A chordoma is a malignant tumor that arises from the embryonic remnants of the notochord (primitive fetal spinal cord). Chordomas represent approximately 1% of all malignant tumors. These tumors can occur anywhere along the spine but approximately 35% are found at the cranial base. A chordoma affects males more that females and can occur at any age. Chordomas present with symptoms, depending on the location, that can include diplopia (double vision), headaches, visual loss, pain, nasal obstruction or discharge, or soft tissue mass. Complete surgical excision is the treatment of choice. If this is not possible, high dose radiation therapy is used.

Choristoma:

Choristomas are congenital lesions that are not true neoplasms. They represent normal tissue in an aberrant location within the body. In the head and neck region, the nasopharynx and the ear represent the most common sites for these very rare lesions. The nasopharyngeal lesions most frequently present as a nasopharyngeal obstruction in the newborn. While these lesions may extend to the cranial base, intracranial communication does not occur. Treatment involves surgical excision.

Craniopharyngioma:

Craniopharyngiomas are benign tumors that arise from the squamous cell nest in the region of the anterior pituitary gland. Common presenting symptoms include visual changes, optic atrophy, hypopituitarism, and headaches. Treatment may include total surgical excision or in the case of large aggressive tumors, partial resection and post operative radiation therapy.

Encephalocele:

An encephalocele is an extracranial herniation of brain and meninges. Encephaloceles may be congenital or acquired through trauma. Some congenital encephaloceles are due to a generalized dysplastic process. Lumbosacral encephaloceles are five times more common than cranial encephaloceles. There is no sex predilection. Presenting symptoms can include meningitis, visible mass, visual changes, headaches, and nasal obstruction. Definitive treatment involves surgical resection.

Epidermoid Carcinoma:

Esthesioneuroblastoma (olfactory neuroblastoma):

Esthesioneuroblastomas are rare, malignant tumors that arise from the epithelium overlying the olfactory bulb. Neuronal information is processed through the bulbs to provide the sense of smell. There are two bulbs, each located high in the nose. Esthesioneuroblastomas are typically slow growing tumors which create few symptoms until they are quite large. The most common presenting symptoms are nasal obstruction, loss of sense of smell, headaches, nosebleeds, visual problems and symptoms of sinusitis. There is no known specific etiology for esthesioneuroblastomas. Most patients are in the fourth, fifth, or sixth decade of life with a slightly higher incidence occurring in women. The current approach to esthesioneuroblastomas is dependent on tumor size and may include craniofacial resection, radiation, chemotherapy, or a combination of treatments.

Fibrosarcoma:

A fibrosarcoma is a malignant tumor of fibroblasts. Fibrosarcomas are found primarily in soft tissues. Approximately 10 to 15% of fibrosarcomas are found in the head and neck region. There is no sex predilection and they may affect all age groups. The most common head and neck sites of fibrosarcomas are the nasal cavity and paranasal sinuses. They may also occur in the larynx and neck. Distal metastases are unusual, but may develop many years after the primary tumor is treated. Symptoms vary according to site but may include nasal obstruction, epistaxis (nosebleed), pain, dysphagia (difficulty swallowing) or the presence of a mass. Treatment involves radical surgical excision. Radiation therapy may be of benefit as well.

Fibrous Dysplasia:

Fibrous dysplasia is a non-neoplastic bone disease in which bone is replaced by fibrous and osseous tissue. There are three variants of fibrous dysplasia which include monostotic, polyostotic and McCune-Albright syndrome. In monostotic fibrous dysplasia only a single osseous site is involved. Seventy percent of the cases of fibrous dysplasia are of the monostotic fibrous dysplasia variety and typically involve the maxilla, mandible, and frontal bones. Polyostotic fibrous dysplasia involves two or more bones. This type represents 25% of all cases of fibrous dysplasia. McCune-Albright syndrome includes a triad of polyostotic fibrous dysplasia, endocrine dysfunction (hyperthyroidism or sexual precocity) and cutaneous hyperpigmentation. Craniofacial fibrous dysplasia represents a third variant of this process. It involves multiple bones of the craniofacial skeleton, but unlike the polyostotic form, does not involve bones outside of the head. Fibrous dysplasia, regardless of type, usually affects patients under the age of thirty. There is no sex predilection in the monostotic variant, but, the polyostotic form affects females more than males. Symptoms of fibrous dysplasia include painless, asymmetric swelling associated with functional disturbances, displacement or malocclusion of teeth, failure of tooth eruption in children, headaches, proptosis (protrusion of the eye), nasal obstruction and hearing loss. Conservative surgical excision is the treatment of choice and is indicated only when there is compromise of function, progression of deformity, pain, associated pathologic fracture or development of a malignancy.

Glioma:

A glioma is a congenital non-neoplastic displacement of brain tissue outside of the cranial cavity without connection to the cranial cavity. A glioma has no sexual predilection. Gliomas are most commonly found near the nasal cavity, ethmoid sinus, palate, middle ear, tonsil or pharynx. They may present with obstruction or the presence of a mass. Surgical excision is the treatment of choice.

Glomus Jugulare Tumor:

A glomus tumor is also called a paraganglioma. Glomus tumors are considered the most common tumor of the middle ear. They affect females more frequently than males. The most common symptom of a glomus tumor is a conductive hearing loss. Glomus tumors can also lead to tinnitus(ringing in the ears), otorrhea (ear drainage), facial nerve abnormalities and vertigo (dizziness). They are often locally invasive tumors. Treatment involves complete surgical excision, but this may be limited by the location and invasive nature of the tumor. Tumors limited to the middle ear are readily removed in a simple operation. Large tumors extending from the brain, through the cranial base, and into the neck, require pre-operative embolization and an extensive surgical procedure for complete resection.

Hemangioma:

Hemangiomas are the most common head and neck neoplasms in children. Although most are found on cutaneous surfaces they may exist on mucosal surfaces as well. They are more common in females than in males. Hemangiomas are a benign, nonreactive process in which an increase in the number of normal and abnormal appearing blood vessels occurs. Several different types of hemangiomas have been described to include: capillary hemangiomas, cavernous hemangiomas and arteriovenous hemangiomas. While capillary hemangiomas are typically superficial, cavernous and arteriovenous hemangiomas frequently involve deeper structures in the head and neck region. In addition, capillary hemangiomas frequently involute while the other two varieties do not. Conservative therapy is the rule for many hemangiomas due to the fact that most congenital lesions involute. However, if a lesion exhibits rapid growth, hemorrhage, recurrent infection, or is in a symptomatic location definitive surgical resection is indicated.

Hemangiopericytoma:

Hemangiopericytomas are uncommon, vasoformative, sarcomatous (malignancy of the soft tissue) tumors. Hemangiopericytomas may occur anywhere in the body with approximately one quarter of tumors occurring in the head and neck region. They are somewhat unpredictable neoplasms with some tumors exhibiting rapid growth while others exhibiting minimal change over years of observation. There appears to be no sexual predilection and only a slightly higher incidence in middle age. Hemangiopericytomas of the cranial base are typically painless lesions, commonly presenting as nasal obstruction with or without nasal bleeding. Treatment of hemangiopericytomas is primarily surgery. Radiation therapy may decrease the size of the tumor but cure with radiation therapy is rare. Distant metastasis to lung, liver, and bone can occur but regional metastasis to cervical lymph nodes is very rare.

Inverting Papilloma:

Inverting papillomas are benign tumors that typically arise from the lateral wall of the nose. Extension to all of the sinuses as well as the cranial base is possible. While inverting papillomas are considered benign tumors, approximately 10% undergo malignant change into squamous cell cancers. For this reason, complete surgical resection is considered the treatment of choice. If complete surgical is not achieved or surgery is not a safe treatment choice for the patient, radiation therapy may be indicated.

Juvenile Nasopharyngeal Angiofibroma (JNA):

Juvenile nasopharyngeal angiofibromas are uncommon, benign, slow growing tumors. JNAs most commonly arise from the sphenopalatine foramen or the posterolateral wall of the roof of the nose. These tumors are difficult to manage because they are typically locally destructive causing invasion into the nasal cavity, maxillary sinuses, the orbit and even the brain. Nasal obstruction, epistaxis (nosebleed), rhinorrhea (nasal drainage), facial swelling, displacement of orbital contents, and headache are the most common presenting symptoms. The etiology of JNAs is not known but they are found almost exclusively in males with the highest prevalence in teenagers and young adults. Treatment of JNAs is primarily surgical, but in some situations radiation therapy may be used.

Lymphangioma:

Lymphangiomas of the cystic hygroma type, are benign, congenital malformations of lymphatic spaces that are divided into three types: capillary, cavernous and cystic. There is no sex predilection. Lymphangiomas are usually present at birth and may occur anywhere in the body. The most common location is in the head and neck region. They usually present as a painless mass but may cause difficulty swallowing, shortness of breath or stridor. Surgical excision is the treatment of choice.

Malignant Fibrous Histiocytoma (MFH):

Malignant fibrous histiocytomas are rare malignant tumors that typically arise out of soft tissue but may form from bone. Malignant fibrous histiocytomas are most commonly found in the extremities but approximately 8% of lesions are found in the craniofacial region. The maxilla (upper jaw) and the mandible (lower jaw) are the most common sites in the head. Approximately 70% of malignant fibrous histiocytomas arise as primary tumors while the other 30% are associated with pre-existing conditions such as prior radiation therapy to the region. Males are affected more commonly than females. The presenting symptoms may include a painful mass or nasal congestion. Treatment for primary malignant fibrous histiocytomas involves surgical resection followed by chemotherapy and radiation therapy. In cases of unresectable radiation induced tumors at presentation, chemotherapy followed by surgical salvage (resection of residual tumor) is the treatment regimen.

Meningioma:

Meningiomas are benign tumors which arise from the arachnoid cells that form the arachnoid villi, which are seen in association with the dural sinuses. Meningiomas comprise between 13 to 18% of all intracranial tumors. Meningiomas are the second most common tumor of the cerebellopontine angle. Meningiomas tend to occur more frequently in females than males. They are most common in the fifth decade of life but can be seen over a wide age range. The presence of a meningioma outside the central nervous system is considered to be ectopic. These ectopic meningiomas are differentiated by their connection to the central nervous system (primary) or without a central nervous system connection (secondary). The most common location for ectopic meningiomas to occur in the head and neck region is the middle ear and the temporal bone. Symptoms may include hearing loss, dysequilibrium, headaches, and cranial nerve abnormalities. The treatment of choice is complete surgical excision. Malignant change rarely occurs.

Meningioma, Olfactory Groove:

Olfactory groove meningiomas are located at the at the nerves of smell in the anterior cranial base. The most common presenting symptoms are a decrease in the sense of smell and visual disturbances. See meningioma for further information.

Meningioma, Petroclival:

Petroclival meningiomas are meningiomas located deep in the cranial base adjacent to the nerves that control eye movement or can be even lower, adjacent to the brainstem. See meningioma for further information.

Nasopharyngeal Carcinoma:

Nasopharyngeal carcinoma is a squamous cell carcinoma arising from the surface epithelium and subtyped into three variants: keratinizing, non-keratinizing, and undifferentiated carcinoma. Overall, nasopharyngeal carcinomas are uncommon in the United States, but account for approximately 18% of cancers in China. They may present as a neck mass, hearing loss, nasal obstruction, epistaxis (nosebleed), pain, or headache. Treatment may be surgical depending on the location or may require radiation therapy.

Neurofibroma:

Neurofibromas are localized, benign peripheral nerve sheath tumors. There is no sex predilection for neurofibromas and they commonly occur in the third or fourth decades of life. Neurofibromas can affect any part of the body and are most commonly seen in the skin and subcutaneous tissue. Symptoms vary according to the site, but they usually present as a painless mass compressing on a vital structure. Simple surgical excision is the treatment of choice and is curative. Multiple neurofibromas are characterized most commonly by von Recklinghausen's disease.

Ossifying Fibroma:

Ossifying fibromas are encapsulated, slow-growing benign fibroosseus neoplasms, composed of fibrous tissue mixed with varying amounts of mature bone. Ossifying fibromas may also be called fibrous osteomas, or osteofibromas. They tend to affect women more then men and can occur over a wide age range, but are frequently seen in the third and fourth decades of life. The most common sites of occurrence are the mandible followed by the maxilla. Lesions that occur in the sinuses or the cranial base tend to be more aggressive. These types of tumors typically do not cause symptoms and are often diagnosed incidentally by radiographic examination. Treatment is surgical excision. Prognosis is excellent after complete excision. A variant of ossifying fibroma is juvenile active ossifying fibroma. These tumors may behave aggressively with local destructive capabilities. Treatment of this variant is complete surgical excision which may necessitate radical procedures.

Osteoblastoma:

Osteoblastomas are rare benign tumors of bone. Osteoblastomas comprise only 1% of all primary bone tumors and less than 5% of osteoblastomas are found in the head and neck region. Most osteoblastomas arise in the second and third decades of life and seem to be twice as prevalent in males. Osteoblastomas often resemble osteogenic sarcomas, osteomas, and aneurysmal bone cysts. Very careful histologic examination may be necessary to make an accurate diagnosis. Primary treatment is surgical resection of the entire lesion when possible. Local recurrence of the tumor is possible. Radiation therapy may also have a role in treatment of osteoblastomas in certain circumstances.

Osteoma:

An osteoma is a benign bone forming tumor. Osteomas occur primarily in the craniofacial skeleton involving the paranasal sinuses, especially the frontal sinus. Osteomas tend to affect men more frequently than women and are most common between the ages of 10 and 25. They can also involve the temporal bones, skull, mandible and maxillas. Osteomas are usually asymptomatic, but can be associated with facial asymmetry, erosion of bone, facial swelling or headache. Osteomas require no treatment unless symptomatic. If symptomatic, then surgical excision is curative.

Osteosarcoma:

Osteosarcomas are also called osteogenic sarcomas. Osteosarcomas are considered malignant tumors that arise from bone. Osteosarcomas are the second most common malignant bone tumor and typically arise in long bones (arms and legs). In the head and neck region they are rare, accounting for only about 6% of all osteogenic sarcomas. The most frequent site in the head and neck is the mandible followed by the maxilla, paranasal sinuses and skull. They are most common in the third or fourth decades of life. Symptoms vary depending upon the site of involvement, but, may include nasal obstruction, epistaxis (nosebleed), facial pain and swelling, and dental disorders. Osteosarcomas can also arise in the setting of Paget's disease (disorder of increased bone resorption), prior radiation therapy, or fibrous dysplasia. Treatment includes complete surgical excision. Radiation and chemotherapy may also be used in conjunction with surgery. Metastases typically occur late in the course of the disease.

Rhabdomyosarcoma:

A rhabdomyosarcoma is a malignant tumor of skeletal muscle. Rhabdomyosarcomas comprise approximately 19% of all sarcomas. They are the most common soft tissue sarcomas occurring in the head and neck region. There is no sex predilection with rhabdomyosarcomas. Excluding the orbit, rhabdomyosarcomas occur most frequently in the nasopharynx, ear and sinonasal cavity. Common symptoms may include nasal congestion, sinus fullness/pain, hypernasal speech, headaches, vision changes, and ear fullness/pain. Treatment may involve the use of radiation therapy, multi-agent chemotherapy and surgery.

Schwannoma, Benign:

Schwannomas can be either benign or malignant tumors of peripheral nerves. Benign schwannomas are also called neurilemmomas. Schwannomas can occur anywhere in the body, but are found most frequently in the head and neck region and the extremities. Schwannomas affect women more frequently than men. They occur most commonly in the third to sixth decades of life. Symptoms vary according to site, but usually the tumor usually presents as a solitary, painless, neck mass. Airway obstruction, voice change or difficulty swallowing are also possible symptoms. Surgical excision is the treatment of choice. Local recurrences are infrequent.

Schwannoma, Malignant:

Malignant schwannoma are malignant tumors of peripheral nerves. Malignant schwannomas are also called neurogenic sarcomas or neurofibrosarcomas. Malignant schwannomas account for approximately 14% of all soft tissue sarcomas in the head and neck region. Malignant schwannomas can occur sporadically of unknown etiology or be associated with von Recklinghausen's disease (neurofibromatosis). The most common site of involvement in the head and neck region is the neck. Symptoms include a neck mass with associated pain, paresthesia and weakness. Complete surgical excision is the treatment of choice, but radiation therapy may be used in conjunction with surgery or in cases of recurrent disease.

Sinonasal Melanoma:

Malignant melanomas may develop from specific cells called melanocytes in the mucosa of the nose and sinuses. The anterior or front part of the nasal septum is the most common location followed by the middle and inferior turbinates which are cartilaginous structures in the nose. Melanomas may also be found arising from the maxillary antrum or opening into the maxillary sinuses. The most common presenting symptom is epistaxis (nosebleed). Other symptoms may include nasal congestion and sinus fullness/pressure. While the incidence of malignant melanomas of all types is approximately two per 100,000 persons per year only 2% of these are found in the nose or sinuses. These malignant lesions are rarely seen prior to puberty, have equal incidence in men and women, and tend to have a worse prognosis when compared to cutaneous melanomas. Metastasis can occur with sinonasal melanomas. Aggressive radical surgical excision is the treatment of choice.

Squamous Cell Carcinoma:

A squamous cell carcinoma is a malignant epithelial neoplasm which arises from the surface epithelium. Squamous cell carcinomas are also called epidermoid carcinomas, nonkeratinizing carcinomas, and transitional cell carcinomas. Squamous cell carcinoma represents the most common type of malignant epithelial neoplasm affecting the head and neck region. It tends to affect men more frequently than women. Squamous cell carcinomas may present as asymmetry of the face, nasal obstruction, a visible mass, a nonhealing sore or ulcer, or proptosis (protrusion of the eye). Therapy includes surgery, radiation therapy, and/or chemotherapy depending on the clinical stage.

Disclaimer
The Cranial Base Program provides online information as a community service. This information is in no way intended to take the place of the advice and recommendations of medical providers. If you have, or suspect you have, a health problem, please contact us or your personal provider.